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Multiple endocrine neoplasia in a 70-year-old woman are described. The findings include a nonfunctioning pituitary adenoma, a multicentric papillary thyroid carcinoma, bilateral carotid body paragangliomas, parathyroid hyperplasia, gastric leiomyoma and systemic amyloidosis. A study of the kindred revealed that two family members, a daughter and a granddaughter, have clinical and radiographic evidence of pituitary tumors and bilateral carotid body paragangliomas, suggesting that this may represent a genetically determined syndrome inherited as an autosomal dominant.